DISEASE AREA COORDINATORS

Prof. Dr. Andreas Günther (UGMLC), PD Dr. Anne Hilgendorff (CPC-M)

(Administr. Coordinator: Dr. Antje Brand (CPC-M))

CONTRIBUTING PARTNER SITES

BREATH, CPC-M, TLRC, UGMLC

OVERVIEW

Diffuse Parenchymal Lung Disease (DPLD) refers to more than 200 different diseases, which are characterized by progressive scarring of the lung architecture and often result in respiratory failure. DPLDs occur in children and adults secondary to acute or chronic lung injury provoked by the inhalation of toxic gases or dusts, as part of systemic diseases, or as a result of therapeutic measures. In a considerable part of the cases, however, the cause remains unknown. Among this group are the Idiopathic Interstitial Pneumonias (IIP), to which the relatively frequent and aggressive form Idiopathic Pulmonary Fibrosis (IPF) belongs. Although different in origin, the course of many DPLDs is similar with regard to the progressive scarring of the lung and the poor prognosis. The only curative treatment is lung transplantation. This Disease Area aims to develop new diagnostic strategies and causal treatment options by gaining deeper insights into the underlying disease mechanisms, from the newborn to the elderly patient.

MAJOR RESEARCH GOALS

  • Precise identification of initial trigger events and aggravating factors, verification of so-called ‘secondary hits’ in pulmonary fibrosis
  • Thorough dissection of early cellular responses during the development of pulmonary fibrosis (e.g., regenerative responses, developmental pathways, cellular plasticity, trafficking and crosstalk, fibroblast activation, and matrix deposition)
  • Exploration of cell-based therapies for treatment of DPLD
  • Continuous acquisition, maintenance, and provision of well-characterized patient data (“deep phenotyping”) via established registries and biobanks
  • Exploration of health economics of DPLD including evaluation of different aspects of patient care and the cost-effectiveness of treatment options
  • Development of novel treatment modalities via initiation and conductance of early clinical trials

CURRENT OBJECTIVES

  • New strategies on clinical phenotyping of pediatric and adult DPLD cohorts
  • Establishment of new clinical and interventional studies for neonatal and adult patients
  • Development of new disease models (in vivo and ex vivo)
  • Development of markers and cellular therapies for initial epithelial damage and fibroproliferation (interactome atlas)

 

DPLD:  Selected Publications from DZL Faculty Members Published Since 2010

Crestani B, Huggins JT, Kaye M, Costabel U, Glaspole I, Ogura T, Song JW, Stansen W, Quaresma M, Stowasser S, Kreuter M. Long-term safety and tolerability of nintedanib in patients with idiopathic pulmonary fibrosis: results from the open-label extension study, INPULSIS-ON. Lancet Respir Med. 2019 Jan;7(1):60-68. doi: 10.1016/S2213-2600(18)30339-4. Epub 2018 Sep 14.
https://www.ncbi.nlm.nih.gov/pubmed/30224318

Prasse A, Binder H, Schupp JC, Kayser G, Bargagli E, Jaeger B, Hess M, Rittinghausen S, Vuga L, Lynn H, Violette S, Jung B, Quast K, Vanaudenaerde B, Xu Y, Hohlfeld JM, Krug N, Herazo-Maya JD, Rottoli P, Wuyts WA, Kaminski N. BAL Cell Gene Expression is Indicative of Outcome and Airway Basal Cell Involvement in IPF. Am J Respir Crit Care Med. 2018 Aug 24. doi: 10.1164/rccm.201712-2551OC.
https://www.ncbi.nlm.nih.gov/pubmed/30141961

Förster K, Sass S, Ehrhardt H, Mous DS, Rottier RJ, Oak P, Schulze A, Flemmer AW, Gronbach J, Hübener C, Desai T, Eickelberg O, Theis FJ, Hilgendorff A. Early Identification of Bronchopulmonary Dysplasia Using Novel Biomarkers by Proteomic Screening. Am J Respir Crit Care Med. 2018 Apr 15; 197(8): 1076-1080. doi: 10.1164/rccm.201706-1218LE.
https://www.ncbi.nlm.nih.gov/pubmed/29053024

Leuschner G, Stocker F, Veit T, Kneidinger N, Winter H, Schramm R, Weig T, Matthes S, Ceelen F, Arnold P, Munker D, Klenner F, Hatz R, Frankenberger M, Behr J, Neurohr C. Outcome of lung transplantation in idiopathic pulmonary fibrosis with previous anti-fibrotic therapy. J Heart Lung Transplant. 2018 Feb; 37(2): 268-274. pii: S1053-2498(17)31886-7. doi: 10.1016/j.healun.2017.07.002.
https://www.ncbi.nlm.nih.gov/pubmed/28734935

Schiller HB, Mayr CH, Leuschner G, Strunz M, Staab-Weijnitz C, Preisendörfer S, Eckes B, Moinzadeh P, Krieg T, Schwartz DA, Hatz RA, Behr J, Mann M, Eickelberg O. Deep Proteome Profiling Reveals Common Prevalence of MZB1-Positive Plasma B Cells in Human Lung and Skin Fibrosis. Am J Respir Crit Care Med. 2017 Nov 15;196(10):1298-1310. doi: 10.1164/rccm.201611-2263OC.
https://www.ncbi.nlm.nih.gov/pubmed/28654764

Burgstaller G, Oehrle B, Gerckens M, White ES, Schiller HB, Eickelberg O. The instructive extracellular matrix of the lung: basic composition and alterations in chronic lung disease. Eur Respir J. 2017 Jul 5; 50(1). pii: 1601805. doi: 10.1183/13993003.01805-2016. Print 2017 Jul. Review.
https://www.ncbi.nlm.nih.gov/pubmed/28679607

Didiasova M, Singh R, Wilhelm J, Kwapiszewska G, Wujak L, Zakrzewicz D, Schaefer L, Markart P, Seeger W, Lauth M, Wygrecka M. Pirfenidone exerts antifibrotic effects through inhibition of GLI transcription factors. FASEB J. 2017 May; 31(5): 1916-1928. doi: 10.1096/fj.201600892RR. Epub 2017 Feb 1.
https://www.ncbi.nlm.nih.gov/pubmed/28148565

El Agha E, Kheirollahi V, Moiseenko A, Seeger W, Bellusci S. Ex vivo analysis of the contribution of FGF10+ cells to airway smooth muscle cell formation during early lung development. Dev Dyn. 2017 Jul; 246(7): 531-538. doi: 10.1002/dvdy.24504. Epub 2017 Jun 1.
https://www.ncbi.nlm.nih.gov/pubmed/28387977

Hess R, Wujak L, Hesse C, Sewald K, Jonigk D, Warnecke G, Fieguth HG, de Maat S, Maas C, Bonella F, Preissner KT, Weiss B, Schaefer L, Kuebler WM, Markart P, Wygrecka M. Coagulation factor XII regulates inflammatory responses in human lungs. Thromb Haemost. 2017 Oct 5; 117(10): 1896-1907. doi: 10.1160/TH16-12-0904. Epub 2017 Aug 17.
https://www.ncbi.nlm.nih.gov/pubmed/28816340

Höppner S, Kinting S, Torrano AA, Schindlbeck U, Bräuchle C, Zarbock R, Wittmann T, Griese M. Quantification of volume and lipid filling of intracellular vesicles carrying the ABCA3 transporter. Biochim Biophys Acta (Molecular Cell Research). 2017 Dec; 1864(12): 2330-2335. doi: 10.1016/j.bbamcr.2017.08.013. Epub 2017 Sep 6.
https://www.ncbi.nlm.nih.gov/pubmed/28887056

Johannson KA, Strâmbu I, Ravaglia C, Grutters JC, Valenzuela C, Mogulkoc N, Luppi F, Richeldi L, Wells AU, Vancheri C, Kreuter M; Erice ILD Working Group. Antacid therapy in idiopathic pulmonary fibrosis: more questions than answers? Lancet Respir Med. 2017 Jul; 5(7): 591-598. doi: 10.1016/S2213-2600(17)30219-9. Review.
https://www.ncbi.nlm.nih.gov/pubmed/28664861

Kreuter M, Wälscher J, Behr J. Antifibrotic drugs as treatment of nonidiopathic pulmonary fibrosis interstitial pneumonias: the time is now (?). Curr Opin Pulm Med. 2017 Sep; 23(5): 418-425. doi: 10.1097/MCP.0000000000000408. Review.
https://www.ncbi.nlm.nih.gov/pubmed/28622199

Mižíková I, Palumbo F, Tábi T, Herold S, Vadász I, Mayer K, Seeger W, Morty RE. Perturbations to lysyl oxidase expression broadly influence the transcriptome of lung fibroblasts. Physiol Genomics. 2017 Aug 1; 49(8): 416-429. doi: 10.1152/physiolgenomics.00026.2017. Epub 2017 Jul 10.
https://www.ncbi.nlm.nih.gov/pubmed/28698228

Ntokou A, Szibor M, Rodríguez-Castillo JA, Quantius J, Herold S, El Agha E, Bellusci S, Salwig I, Braun T, Voswinckel R, Seeger W, Morty RE, Ahlbrecht K. A novel mouse Cre-driver line targeting Perilipin 2-expressing cells in the neonatal lung. Genesis. 2017 Dec; 55(12). doi: 10.1002/dvg.23080. Epub 2017 Oct 27.
https://www.ncbi.nlm.nih.gov/pubmed/29045046

Pozarska A, Rodríguez-Castillo JA, Surate Solaligue DE, Ntokou A, Rath P, Mižíková I, Madurga A, Mayer K, Vadász I, Herold S, Ahlbrecht K, Seeger W, Morty RE. Stereological monitoring of mouse lung alveolarization from the early postnatal period to adulthood. Am J Physiol Lung Cell Mol Physiol. 2017 Jun 1; 312(6): L882-L895. doi: 10.1152/ajplung.00492.2016. Epub 2017 Mar 17.
https://www.ncbi.nlm.nih.gov/pubmed/28314804

Surate Solaligue DE, Rodríguez-Castillo JA, Ahlbrecht K, Morty RE. Recent advances in our understanding of the mechanisms of late lung development and bronchopulmonary dysplasia. Am J Physiol Lung Cell Mol Physiol. 2017 Dec 1; 313(6): L1101-L1153. doi: 10.1152/ajplung.00343.2017. Epub 2017 Sep 28. Review.
https://www.ncbi.nlm.nih.gov/pubmed/28971976

Tan XH, Zheng XM, Yu LX, He J, Zhu HM, Ge XP, Ren XL, Ye FQ, Bellusci S, Xiao J, Li XK, Zhang JS. Fibroblast growth factor 2 protects against renal ischaemia/reperfusion injury by attenuating mitochondrial damage and proinflammatory signalling. J Cell Mol Med. 2017 Nov; 21(11): 2909-2925. doi: 10.1111/jcmm.13203. Epub 2017 May 24.
https://www.ncbi.nlm.nih.gov/pubmed/28544332

Walsh SLF, Maher TM, Kolb M, Poletti V, Nusser R, Richeldi L, Vancheri C, Wilsher ML, Antoniou KM, Behr J, Bendstrup E, Brown K, Calandriello L, Corte TJ, Cottin V, Crestani B, Flaherty K, Glaspole I, Grutters J, Inoue Y, Kokosi M, Kondoh Y, Kouranos V, Kreuter M, Johannson K, Judge E, Ley B, Margaritopoulos G, Martinez FJ, Molina-Molina M, Morais A, Nunes H, Raghu G, Ryerson CJ, Selman M, Spagnolo P, Taniguchi H, Tomassetti S, Valeyre D, Wijsenbeek M, Wuyts W, Hansell D, Wells A; IPF Project Consortium. Diagnostic accuracy of a clinical diagnosis of idiopathic pulmonary fibrosis: an international case-cohort study. Eur Respir J. 2017 Aug 31; 50(2). pii: 1700936. doi: 10.1183/13993003.00936-2017. Print 2017 Aug.
https://www.ncbi.nlm.nih.gov/pubmed/28860269

Behr J, Bendstrup E, Crestani B, Günther A, Olschewski H, Sköld CM, Wells A, Wuyts W, Koschel D, Kreuter M, Wallaert B, Lin CY, Beck J, Albera C. Safety and tolerability of acetylcysteine and pirfenidone combination therapy in idiopathic pulmonary fibrosis: a randomised, double-blind, placebo-controlled, phase 2 trial. Lancet Respir Med. 2016 Jun; 4(6): 445-53. doi: 10.1016/S2213-2600(16)30044-3. Epub 2016 May 5.
https://www.ncbi.nlm.nih.gov/pubmed/27161257

Bonella F, Long X, Ohshimo S, Horimasu Y, Griese M, Guzman J, Kohno N, Costabel U. MUC1 gene polymorphisms are associated with serum KL-6 levels and pulmonary dysfunction in pulmonary alveolar proteinosis. Orphanet J Rare Dis. 2016 Apr 23; 11: 48. doi: 10.1186/s13023-016-0430-2.
https://www.ncbi.nlm.nih.gov/pubmed/27108412

Bush A, Barbato A, Clement A, Cunningham S, Blic Jd, Gilbert C, Goldbeck L, Kiper N, Schwerk N, Griese M. European idiopathic pulmonary fibrosis Patient Charter: a missed opportunity. Eur Respir J. 2016 Jul; 48(1): 282-3. doi: 10.1183/13993003.00505-2016.
https://www.ncbi.nlm.nih.gov/pubmed/27365515

Campo I, Luisetti M, Griese M, Trapnell BC, Bonella F, Grutters JC, Nakata K, Van Moorsel CH, Costabel U, Cottin V, Ichiwata T, Inoue Y, Braschi A, Bonizzoni G, Iotti GA, Tinelli C, Rodi G; WLL International Study Group. A Global Survey on Whole Lung Lavage in Pulmonary Alveolar Proteinosis. Chest. 2016 Jul; 150(1): 251-3. doi: 10.1016/j.chest.2016.04.030.
https://www.ncbi.nlm.nih.gov/pubmed/27396783

Campo I, Luisetti M, Griese M, Trapnell BC, Bonella F, Grutters J, Nakata K, Van Moorsel CH, Costabel U, Cottin V, Ichiwata T, Inoue Y, Braschi A, Bonizzoni G, Iotti GA, Tinelli C, Rodi G; WLL International Study Group. Whole lung lavage therapy for pulmonary alveolar proteinosis: a global survey of current practices and procedures. Orphanet J Rare Dis. 2016 Aug 31; 11(1): 115. doi: 10.1186/s13023-016-0497-9.
https://www.ncbi.nlm.nih.gov/pubmed/27577926

Clement A, de Blic J, Epaud R, Galeron L, Nathan N, Hadchouel A, Barbato A, Snijders D, Kiper N, Cunningham S, Griese M, Bush A, Schwerk N; chILD-EU collaboration. Management of children with interstitial lung diseases: the difficult issue of acute exacerbations. Eur Respir J. 2016 Dec; 48(6): 1559-1563. doi: 10.1183/13993003.01900-2016.
https://www.ncbi.nlm.nih.gov/pubmed/27903688

Collard HR, Ryerson CJ, Corte TJ, Jenkins G, Kondoh Y, Lederer DJ, Lee JS, Maher TM, Wells AU, Antoniou KM, Behr J, Brown KK, Cottin V, Flaherty KR, Fukuoka J, Hansell DM, Johkoh T, Kaminski N, Kim DS, Kolb M, Lynch DA, Myers JL, Raghu G, Richeldi L, Taniguchi H, Martinez FJ. Acute Exacerbation of Idiopathic Pulmonary Fibrosis. An International Working Group Report. Am J Respir Crit Care Med. 2016 Aug 1; 194(3): 265-75. doi: 10.1164/rccm.201604-0801CI. Review.
https://www.ncbi.nlm.nih.gov/pubmed/27299520

Ehrhardt H, Pritzke T, Oak P, Kossert M, Biebach L, Förster K, Koschlig M, Alvira CM, Hilgendorff A. Absence of TNF-α enhances inflammatory response in the newborn lung undergoing mechanical ventilation. Am J Physiol Lung Cell Mol Physiol. 2016 May 15; 310(10): L909-18. doi: 10.1152/ajplung.00367.2015. Epub 2016 Mar 25.
https://www.ncbi.nlm.nih.gov/pubmed/27016588

Fernandez IE, Amarie OV, Mutze K, Königshoff M, Yildirim AÖ, Eickelberg O. Systematic phenotyping and correlation of biomarkers with lung function and histology in lung fibrosis. Am J Physiol Lung Cell Mol Physiol. 2016 May 15; 310(10): L919-27. doi: 10.1152/ajplung.00183.2015. Epub 2016 Mar 18.
https://www.ncbi.nlm.nih.gov/pubmed/26993522

Fernandez IE, Greiffo FR, Frankenberger M, Bandres J, Heinzelmann K, Neurohr C, Hatz R, Hartl D, Behr J, Eickelberg O. Peripheral blood myeloid-derived suppressor cells reflect disease status in idiopathic pulmonary fibrosis. Eur Respir J. 2016 Oct; 48(4): 1171-1183. doi: 10.1183/13993003.01826-2015. Epub 2016 Sep 1.
https://www.ncbi.nlm.nih.gov/pubmed/27587556

Frixel S, Lotz-Havla AS, Kern S, Kaltenborn E, Wittmann T, Gersting SW, Muntau AC, Zarbock R, Griese M. Homooligomerization of ABCA3 and its functional significance. Int J Mol Med. 2016 Aug; 38(2): 558-66. doi: 10.3892/ijmm.2016.2650. Epub 2016 Jun 21.
https://www.ncbi.nlm.nih.gov/pubmed/27352740

Ge L, Habiel DM, Hansbro PM, Kim RY, Gharib SA, Edelman JD, Königshoff M, Parimon T, Brauer R, Huang Y, Allen J, Jiang D, Kurkciyan AA, Mizuno T, Stripp BR, Noble PW, Hogaboam CM, Chen P. miR-323a-3p regulates lung fibrosis by targeting multiple profibrotic pathways. JCI Insight. 2016 Dec 8; 1(20): e90301.
https://www.ncbi.nlm.nih.gov/pubmed/27942594

Heinzelmann K, Noskovičová N, Merl-Pham J, Preissler G, Winter H, Lindner M, Hatz R, Hauck SM, Behr J, Eickelberg O. Surface proteome analysis identifies platelet derived growth factor receptor-alpha as a critical mediator of transforming growth factor-beta-induced collagen secretion. Int J Biochem Cell Biol. 2016 May; 74: 44-59. doi: 10.1016/j.biocel.2016.02.013. Epub 2016 Feb 22.
https://www.ncbi.nlm.nih.gov/pubmed/26905437

Hellbach K, Yaroshenko A, Willer K, Pritzke T, Baumann A, Hesse N, Auweter S, Reiser MF, Eickelberg O, Pfeiffer F, Hilgendorff A, Meinel FG. Facilitated Diagnosis of Pneumothoraces in Newborn Mice Using X-ray Dark-Field Radiography. Invest Radiol. 2016 Oct; 51(10): 597-601. doi: 10.1097/RLI.0000000000000285.
https://www.ncbi.nlm.nih.gov/pubmed/27603110

Hofmann N, Galetskiy D, Rauch D, Wittmann T, Marquardt A, Griese M, Zarbock R. Analysis of the Proteolytic Processing of ABCA3: Identification of Cleavage Site and Involved Proteases. PLoS One. 2016 Mar 31; 11(3): e0152594. doi: 10.1371/journal.pone.0152594. eCollection 2016.
https://www.ncbi.nlm.nih.gov/pubmed/27031696

Horowitz JC, Osterholzer JJ, Marazioti A, Stathopoulos GT. "Scar-cinoma": viewing the fibrotic lung mesenchymal cell in the context of cancer biology. Eur Respir J. 2016 Jun; 47(6): 1842-54. doi: 10.1183/13993003.01201-2015. Epub 2016 Mar 30.
https://www.ncbi.nlm.nih.gov/pubmed/27030681

Behr J. Guidelines or guidance for better idiopathic pulmonary fibrosis management? BMC Med. 2016 Feb 10; 14: 24. doi: 10.1186/s12916-016-0567-9.
https://www.ncbi.nlm.nih.gov/pubmed/26860906

Klee S, Lehmann M, Wagner DE, Baarsma HA, Königshoff M. WISP1 mediates IL-6-dependent proliferation in primary human lung fibroblasts. Sci Rep. 2016 Feb 12; 6: 20547. doi: 10.1038/srep20547.
https://www.ncbi.nlm.nih.gov/pubmed/26867691

Kolahian S, Fernandez IE, Eickelberg O, Hartl D. Immune Mechanisms in Pulmonary Fibrosis. Am J Respir Cell Mol Biol. 2016 Sep; 55(3): 309-22. doi: 10.1165/rcmb.2016-0121TR. Review.
https://www.ncbi.nlm.nih.gov/pubmed/27149613

Kröner C, Wittmann T, Reu S, Teusch V, Klemme M, Rauch D, Hengst M, Kappler M, Cobanoglu N, Sismanlar T, Aslan AT, Campo I, Proesmans M, Schaible T, Terheggen-Lagro S, Regamey N, Eber E, Seidenberg J, Schwerk N, Aslanidis C, Lohse P, Brasch F, Zarbock R, Griese M. Lung disease caused by ABCA3 mutations. Thorax. 2017 Mar; 72(3): 213-220. doi: 10.1136/thoraxjnl-2016-208649. Epub 2016 Aug 11.
https://www.ncbi.nlm.nih.gov/pubmed/27516224

Lehmann M, Baarsma HA, Königshoff M. WNT Signaling in Lung Aging and Disease. Ann Am Thorac Soc. 2016 Dec; 13(Supplement_5): S411-S416. doi: 10.1513/AnnalsATS.201608-586AW. Review.
https://www.ncbi.nlm.nih.gov/pubmed/28005418

Leuschner G, Wenter V, Milger K, Zimmermann GS, Matthes S, Meinel FG, Lehner S, Neurohr C, Behr J, Kneidinger N. Suspected pulmonary embolism in patients with pulmonary fibrosis: Discordance between ventilation/perfusion SPECT and CT pulmonary angiography. Respirology. 2016 Aug; 21(6): 1081-7. doi: 10.1111/resp.12797. Epub 2016 Apr 7.
https://www.ncbi.nlm.nih.gov/pubmed/27061739

Long X, He X, Ohshimo S, Griese M, Sarria R, Guzman J, Costabel U, Bonella F. Serum YKL-40 as predictor of outcome in hypersensitivity pneumonitis. Eur Respir J. 2017 Feb 23; 49(2). pii: 1501924. doi: 10.1183/13993003.01924-2015. Print 2017 Feb.
https://www.ncbi.nlm.nih.gov/pubmed/27836954

Manali ED, Papadaki G, Konstantonis D, Tsangaris I, Papaioannou AI, Kolilekas L, Schams A, Kagouridis K, Karakatsani A, Orfanos S, Griese M, Papiris SA. Cardiovascular risk in pulmonary alveolar proteinosis. Expert Rev Respir Med. 2016 Feb; 10(2): 235-40. doi: 10.1586/17476348.2016.1116389. Epub 2015 Nov 27.
https://www.ncbi.nlm.nih.gov/pubmed/26558331

Meiners S, Hilgendorff A. Early injury of the neonatal lung contributes to premature lung aging: a hypothesis. Mol Cell Pediatr. 2016 Dec; 3(1): 24. doi: 10.1186/s40348-016-0052-8. Epub 2016 Jul 12. Review.
https://www.ncbi.nlm.nih.gov/pubmed/27406259

Ota C, Baarsma HA, Wagner DE, Hilgendorff A, Königshoff M. Linking bronchopulmonary dysplasia to adult chronic lung diseases: role of WNT signaling. Mol Cell Pediatr. 2016 Dec; 3(1): 34. Epub 2016 Oct 7. Review.
https://www.ncbi.nlm.nih.gov/pubmed/27718180

Pereira PR, Oliveira-Junior MC, Mackenzie B, Chiovatto JE, Matos Y, Greiffo FR, Rigonato-Oliveira NC, Brugemman TR, Delle H, Idzko M, Albertini R, Ligeiro Oliveira AP, Damaceno-Rodrigues NR, Caldini EG, Fernandez IE, Castro-Faria-Neto HC, Dolhnikoff M, Eickelberg O, Vieira RP. Exercise Reduces Lung Fibrosis Involving Serotonin/Akt Signaling. Med Sci Sports Exerc. 2016 Jul; 48(7): 1276-84. doi: 10.1249/MSS.0000000000000907.
https://www.ncbi.nlm.nih.gov/pubmed/26895395

Rauch D, Wetzke M, Reu S, Wesselak W, Schams A, Hengst M, Kammer B, Ley-Zaporozhan J, Kappler M, Proesmans M, Lange J, Escribano A, Kerem E, Ahrens F, Brasch F, Schwerk N, Griese M; PTI (Persistent Tachypnea of Infancy) Study Group of the Kids Lung Register. Persistent Tachypnea of Infancy. Usual and Aberrant. Am J Respir Crit Care Med. 2016 Feb 15; 193(4): 438-47. doi: 10.1164/rccm.201508-1655OC.
https://www.ncbi.nlm.nih.gov/pubmed/26474448

Schamberger AC, Schiller HB, Fernandez IE, Sterclova M, Heinzelmann K, Hennen E, Hatz R, Behr J, Vašáková M, Mann M, Eickelberg O, Staab-Weijnitz CA. Glutathione peroxidase 3 localizes to the epithelial lining fluid and the extracellular matrix in interstitial lung disease. Sci Rep. 2016 Jul 20; 6: 29952. doi: 10.1038/srep29952.
https://www.ncbi.nlm.nih.gov/pubmed/27435875

Smirnova NF, Schamberger AC, Nayakanti S, Hatz R, Behr J, Eickelberg O. Detection and quantification of epithelial progenitor cell populations in human healthy and IPF lungs. Respir Res. 2016 Jul 16; 17(1): 83. doi: 10.1186/s12931-016-0404-x.
https://www.ncbi.nlm.nih.gov/pubmed/27423691

Spanjer AI, Baarsma HA, Oostenbrink LM, Jansen SR, Kuipers CC, Lindner M, Postma DS, Meurs H, Heijink IH, Gosens R, Königshoff M. TGF-β-induced profibrotic signaling is regulated in part by the WNT receptor Frizzled-8. FASEB J. 2016 May; 30(5): 1823-35. doi: 10.1096/fj.201500129. Epub 2016 Feb 5.
https://www.ncbi.nlm.nih.gov/pubmed/26849959

Staab-Weijnitz CA, Eickelberg O. Repositioning compounds from cancer drug discovery to IPF: PI3K inhibition. Thorax. 2016 Aug; 71(8): 675-6. doi: 10.1136/thoraxjnl-2016-208680. Epub 2016 Jun 15.
https://www.ncbi.nlm.nih.gov/pubmed/27307018

Sun N, Fernandez IE, Wei M, Wu Y, Aichler M, Eickelberg O, Walch A. Pharmacokinetic and pharmacometabolomic study of pirfenidone in normal mouse tissues using high mass resolution MALDI-FTICR-mass spectrometry imaging. Histochem Cell Biol. 2016 Feb; 145(2): 201-11. doi: 10.1007/s00418-015-1382-7. Epub 2015 Dec 8.
https://www.ncbi.nlm.nih.gov/pubmed/26645566

White ES, Borok Z, Brown KK, Eickelberg O, Guenther A, Jenkins RG, Kolb M, Martinez FJ, Roman J, Sime P; American Thoracic Society Respiratory Cell and Molecular Biology Assembly Working Group on Pulmonary Fibrosis. An American Thoracic Society Official Research Statement: Future Directions in Lung Fibrosis Research. Am J Respir Crit Care Med. 2016 Apr 1; 193(7): 792-800. doi: 10.1164/rccm.201602-0254ST.
https://www.ncbi.nlm.nih.gov/pubmed/27035782

Wittmann T, Frixel S, Höppner S, Schindlbeck U, Schams A, Kappler MV, Hegermann J, Wrede C, Liebisch G, Vierzig A, Zacharasiewicz A, Kopp M, Poets CF, Baden W, Hartl D, Van Kaam AH, Lohse P, Aslanidis C, Zarbock R, Griese M. Increased risk of interstitial lung disease in children with a single R288K variant of ABCA3. Mol Med. 2016; 22: 183-191. doi: 10.2119/molmed.2015.00244. Epub 2016 Feb 26.
https://www.ncbi.nlm.nih.gov/pubmed/26928390

Wittmann T, Schindlbeck U, Höppner S, Kinting S, Frixel S, Kröner C, Liebisch G, Hegermann J, Aslanidis C, Brasch F, Reu S, Lasch P, Zarbock R, Griese M. Tools to explore ABCA3 mutations causing interstitial lung disease. Pediatr Pulmonol. 2016 Dec; 51(12): 1284-1294. doi: 10.1002/ppul.23471. Epub 2016 May 13.
https://www.ncbi.nlm.nih.gov/pubmed/27177387

Yaroshenko A, Pritzke T, Koschlig M, Kamgari N, Willer K, Gromann L, Auweter S, Hellbach K, Reiser M, Eickelberg O, Pfeiffer F, Hilgendorff A. Visualization of neonatal lung injury associated with mechanical ventilation using x-ray dark-field radiography. Sci Rep. 2016 Apr 13; 6: 24269. doi: 10.1038/srep24269.
https://www.ncbi.nlm.nih.gov/pubmed/27072871